What is epidermolysis bullosa?

Skin is a pretty amazing organ. It's in a constant state of death and rebirth as new skin cells (called keratinocytes) are born and replace the dead cells that slough off. Before they're lost, dead skin cells serve as the barrier that protects the younger skin and internal organs beneath from exposure to the elements, radiation from the sun and infection from bacteria.

Human skin also has a defense system that goes to work when its integrity gets compromised by injuries from friction or heat. You see this system in action every time a blister forms on your skin.

When skin is irritated by friction or exposure to high temperatures, the layers can loosen. When this occurs, the empty pocket between them is filled with a fluid called serum. This serum serves as a cushion that allows the immature, tender skin beneath to heal, a process called re-epithelialization. As it does, the injured outside layer deadens and falls off.

Blisters are a normal and beneficial reaction to damaged skin. For most of us, they pose little more than a nuisance. For people who suffer from a condition called epidermolysis bullosa, however, blisters can be life-threatening.

Epidermolysis bullosa (EB) is a disorder where inherited mutations on 10 genes lead to a heightened blister response in the sufferer's skin. People with EB have fragile skin; even mild heat and friction can create injuries and cause blisters. While blisters can be painful, consistent and prolonged blistering can also pose a risk to a person's health, as it increases the likelihood that a blister will become infected [source: NIAMS].

The condition is actually a set of varying conditions that are defined by how blisters form, but all EBs share a lack of a cure.

On the next page we'll look at different types of EB and how sufferers deal with their condition.